1例肝豆状核变性所致精神障碍患者的护理

肝豆状核变性又称Wilson病,为常染色体隐性遗传的铜代谢障碍所致家族性疾病.起病大多在青少年期[1],病变主要累及肝脏和以豆状核等基底节为主的中枢神经系统[2].临床主要表现以肢体震颤、肌强直、构音不清、精神障碍、肝硬化为特征[3].本病所致精神障碍症状复杂多样,易误诊,病情发展缓慢,目前尚无特效药物治疗.我院2005年曾收治1例肝豆状核变性所致精神障碍的患者,经精心治疗和护理,病情稳定出院,现将护理体会报道如下:     

肝豆状核变性伴发精神障碍的临床分析

目的：探讨肝豆状核变性伴发精神障碍的临床特点及误诊原因。方法：对23例患者进行K－F环和血清铜氧化酶活力检查。结果：23例K－F环阳性，4例血清铜氧化酶活力下降，22例误诊。结论：对有精神障碍的肝豆状核变性患者应进行全面检查，以减少误诊，并应配合小剂量抗精神病药物治疗。     

1例肝豆状核变性所致精神障碍患者的护理

肝豆状核变性又称Wilson病,为常染色体隐性遗传的铜代谢障碍所致家族性疾病。起病大多在青少年期[1],病变主要累及肝脏和以豆状核等基底节为主的中枢神经系统[2]。临床主要表现以肢体震颤、肌强直、构音不清、精神障碍、肝硬化为特征[3]。本病所致精神障碍症状复杂多样,易误诊,病情发展缓慢,目前尚无特效药物治疗。我院2005年曾收治1例肝豆状核变性所致精神障碍的患者,经精心治疗和护理,病情稳定出院,现将护理体会报道如下:1病例介绍患者,男,22岁,因猜疑,胡言乱语,睡眠差,情绪易激惹,有冲动行为且有明显进食困难,生活需他人协助料理而收住…     

驱铜治疗及护理干预对肝豆状核变性患者的疗效观察

目的 观察驱铜治疗及护理干预对肝豆状核变性患者的疗效.方法 根据治疗情况,分为疗前组、短期治疗组及长期治疗组；采用药物治疗的同时,给予护理干预,比较各组间临床症状评分有无差异.结果 短期治疗组症状评分与疗前组比较差异无显著意义,长期治疗组症状评分与疗前组比较明显减低(P＜0.01).结论 在常规驱铜治疗的基础上,针对患者的临床特点,给予相应的护理干预,可促进患者的临床症状恢复.     

1例肝豆状核变性伴精神障碍病人烧伤的护理

肝豆状核变性是一种常染色体隐性遗传疾病,因机体铜代谢障碍,肝脏出现进行性豆状核变性及肝硬化等为主的病理性改变,严重者可出现神经系统病变.其症状为进行性加重的肢体震颤、肌强直、构音困难等锥体外系表现和肝功能损坏及角膜色素环等.发病年龄多在7岁～25岁,男性多于女性[1].我科于2009年3月25日收治1例肝豆状核变性伴精神障碍且烧伤的病人,经精心治疗和护理,烧伤痊愈出院,现将护理体会报道如下.     

1例肝豆状核变性致严重椎体外系症状患者的护理

肝豆状核变性是一种常染色体隐性遗传铜代谢障碍疾病。主要累及脑基底节及肝、肾、角膜等器官而出现一系列临床症状。临床上,以锥体外系症状为首发症状者较多见,精神症状为首发症状者约占20％,易误诊为原发性精神病。我科2002年7月收治1例2次被误诊为精神分裂症,服用大量抗精神病药后以严重锥体外系症状为主要表现入院的肝豆状核变性患者,经仔细的观察和精心的护理取得较好的疗效,现将护理体会报告如下。     

肝豆状核变性的护理

总结了12例肝豆状核变性的护理经验，包括：饮食治疗，驱铜药物治疗观察护理，心理护理，出院指导，取得了较好的临床效果，该善了患者的生活质量。     

肝豆状核变性伴发精神障碍1例的健康教育

为了更好护理肝豆状核变性伴发精神障碍的患者,对我科肝豆状核变性伴发精神障碍患者的护理问题进行有针对性的健康教育及护理干预,效果满意,现报告如下.1病历摘要女,26岁,已婚.主因间断情绪不稳,兴奋话多19a,复发半月,缘于8岁时患者出现流口水、走路不稳摔倒,梳头时拿不住梳子,学习差,情绪尚可.诊断肝豆状核变性后坚持服用青霉胺、葡萄糖酸锌片等治疗,能正常学习,继续服药,近半个月情绪不稳,打骂母亲,兴奋话多,烦躁.检查:角膜可见K-F环,脑CT示双豆状核低密度影.患者肝功能未见异常.检查铜蓝蛋白偏低(2.33 mg/dl),血清转铁蛋白(163 mg/dl)长期服用青霉胺、葡萄糖酸锌治疗.给予奥氮平片和丙戊酸纳缓释片口服治疗,经治疗护理,患者病情稳定,好转出院.     

肝豆状核变性脾切除的治疗与探讨(附54例报告)

目的 探讨遗传性疾病肝豆状板变性合并严重脾功能亢进患者的有效治疗方法。方法 对54例肝豆状核变性合并脾亢而行脾切除的患者进行分析，比较手术前后外周血细胞变化。结果 全部患者术后白细胞及血小板显著增高，而神经症状基本未加重，患者能继续驱铜治疗。结论 脾切除疗效满意．是肝豆状核变性合并严重脾功能亢进的重要辅助治疗措施.     

吞咽治疗仪治疗肝豆状核变性患者吞咽障碍的疗效观察

目的 :观察德国菲兹曼吞咽治疗仪治疗肝豆状核变性患者吞咽障碍疗效。方法:将60例肝豆状核变性伴吞咽障碍的患者随机分为2组,在驱铜治疗的基础上,实验组采用吞咽治疗仪辅助康复的训练,对照组仅采用驱铜治疗。结果 :治疗后2组均能改善吞咽障碍,实验组疗效优于对照组(P0.05)。结论 :吞咽障碍治疗仪治疗肝豆状核变性吞咽障碍疗效显著,能有效改善患者的吞咽功能,提高临床疗效,减少营养不良、吸入性肺炎及窒息等并发症的发生。     

中药肝豆汤对遗传性肝豆状核变性患者铜锌代谢的影响

目的在细胞水平探讨中药复方肝豆汤治疗肝豆状核变性(HLD)的机制。方法在以HLD患者的肝脏制备的肝细胞模型中加入含肝豆汤兔血清,观察加入前后细胞模型中铜锌等微量元素含量的变化。结果加入含肝豆汤兔血清24h后细胞内铜含量明显减少,锌含量明显增加。结论肝豆汤具有显著的细胞内排铜作用,同时可使细胞内锌含量显著增加。     

Reflections on Gallstones

The longer an individual harbors gallstones, the greater the incidence of serious complications. The most common complications are acute cholecystitis, pancreatitis and a stone in the common duct; morbidity and mortality tend to double in patients more than 60 years old. Hopefully, research will lead to effective control of gallstones by diet or medication, or both. Lecithin shows promise in dissolving stones and preventing formation of new ones.     

Nonconvulsive Status Epilepticus in Theophylline Toxicity

Case Report: A 53-year-old cocaine user was on chronic therapy with theophylline, albuterol, and ipratropium for asthma and nifedipine for hypertension. Acute asthma treatment that increased the serum theophylline to 35 μg/mL was associated with tonic clonic seizures followed by bizarre, lateralized posturing. Electroencephalogram seizure activity lasting 10 days was consistent with nonconvulsive status epilepticus, Complex Partial, type II. Theophylline was considered the probable instigator of this underdiagnosed neurologic disorder.     

Copper Biology in Health and Disease: Copper in the tumor microenvironment and tumor metastasis

Copper (Cu), an essential micronutrient, plays an essential role in several physiological processes, including cell proliferation and angiogenesis; however, its dysregulation induces oxidative stress and inflammatory responses. Significant Cu accumulation is observed in several tumor tissues. The bioavailability of intracellular Cu is tightly controlled by Cu transporters, including Cu transporter 1 (CTR1) and Cu-transporting P-type ATPase α and β (ATP7A and ATP7B), and Cu chaperones, including Cu chaperone for superoxide dismutase 1 (CCS) and antioxidant-1 (Atox-1). In several tumor tissues, these abnormalities that induce intra­cellular Cu accumulation are involved in tumor progression. In addition, functional disturbance in Cu-containing secretory enzymes, such as superoxide dismutase 3 (SOD3), and lysyl oxidase enzymes (LOX and LOXL1–4) with abnormal Cu dynamics plays a key role in tumor metastasis. For example, the loss of SOD3 in tumor tissues induces oxidative stress, which promotes neovascularization and epithelial-to-mesenchymal transition (EMT). LOX promotes collagen crosslinking, which functions in the metastatic niche formation. Accordingly, restricted Cu regulation may be a novel strategy for the inhibition of tumor metastasis. However, it is unclear how these Cu disturbances occur in tumor tissues and the exact molecular mechanisms underlying Cu secretory enzymes. In this review article, I discuss the role of Cu transporters, Cu chaperones, and Cu-containing secretory enzymes in tumor progression to better understand the role of Cu homeostasis in tumor tissues.     

情志护理在脑卒中患者心理和情感障碍康复中的应用

情志护理是以中医基础理论为指导,以良好的护患关系为桥梁,应用科学的护理方法,改善和消除患者的不良情绪状态,从而达到预防和治疗疾病目的的一种方法。脑卒中是临床常见病及多发病,为目前人类三大死亡病因之一,并且是最主要的致残因素。患者患病后生活不能自理,心理压力大,严重影响了疾病的康复及生存质量。     

The Child With Recurrent Infections

The child who has a history of recurrent infections may have any of a wide variety of diseases caused by defective functioning of the host's defenses. Clinical manifestations depend on a quantitative deficiency or a qualitative functional disturbance of an immunologically important cell or organ. These disorders are potentially disabling and often life-threatening; thus, the physician must be aware of the features of the presently known immunologic disorders.     

首诊抑郁症2年转归及影响因素

目的探讨抑郁症诊断、转归及其影响因素。方法对111例抑郁症病人首次诊断、病程1年及病程2年临床资料进行回顾性分析,结合其住院记录和病程记录,根据CCMD-3诊断标准进行再诊断,并应用汉密尔顿抑郁量表（HAMD）进行评分。结果病程1年时,维持抑郁症诊断者83例（74．8％）,转归为双相障碍者25例（22．5％）,转归为精神分裂症、分裂情感类障碍、分离转换性障碍者各1例（分别占0．1％）。病程2年时,维持抑郁症诊断者75例（67．6％）,转归为双相障碍者29例（26．1％）,其余诊断未改变。发病年龄、家族史及诱发因素是抑郁症向双相障碍转归的显著影响因子。结论抑郁症在2年病程内多转归为双相障碍,发病年龄、家族史、诱发因素可以预测其转归为双相障碍。     

The prevalence of migraine in patients with bipolar and unipolar affective disorders

There is a well-known association between migraine and affective disorders, but the information is sparse concerning the prevalence of migraine in subgroups of the affective disorders. The present study was undertaken to investigate the prevalence of migraine in unipolar depressive, bipolar I and bipolar II disorders. Patients with major affective disorders (n = 62), consecutively admitted to an open psychiatric ward, were examined with a semi-structured interview based on DSM-IV diagnostic criteria, combined with separate criteria for affective temperaments. Diagnosis of unipolar and bipolar I disorders followed the DSM-IV criteria, while bipolar II disorder encompassed patients with either discrete hypomanic episodes or a cyclothymic temperament. Migraine was diagnosed according to IHS-criteria. Symptoms of migraine were found to be common in these patients, both in those with unipolar depression (46% prevalence of migraine) and in those with bipolar disorders (44% prevalence). Among the bipolar patients there was, however, a striking difference between the two diagnostic subgroups, with a prevalence of 77% in the bipolar II group compared with 14% in the bipolar I group (P = 0.001). These results support the contention that bipolar I and II are biologically separate disorders and point to the possibility of using the association of bipolar II disorder with migraine to study both the pathophysiology and the genetics of this affective disorder.     

Risperidone in Children and Adolescents with Conduct Disorder: A Single-Center, Open-Label Study

Background: Risperidone is one of the most commonly used atypical antipsychotic drugs in the treatment of children and adolescents. However, the data about its use in children and adolescents with conduct disorder (CD) are limited.Objective: The aim of this study was to investigate the effectiveness and tolerability of risperidone in controlling major symptoms of CD in children and adolescents diagnosed with attention deficit hyperactivity disorder (ADHD), oppositional defiant disorder (ODD), and severe CD.Methods: Children and adolescents were eligible for this single-center, open-label study if they met the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) diagnostic criteria for ADHD and ODD and also were diagnosed with severe CD. The patients were treated with risperidone in an open-label fashion for 8 weeks, starting at a daily dosage of 0.25 mg or 0.5 mg (depending on their body weight) in 2 divided doses.Results: The study population comprised 21 children and adolescents (17 boys, 4 girls) with a mean (SD) age of 10.8 (3.6) years. The mean (SD) dosage of risperidone at the end of 8 weeks of treatment was 1.27 (0.42) mg/d (range, 0.75-2.0 mg/d). On the basis of the global improvement subscale of the Clinical Global Impression scale, 16 of 20 patients (80%) were classified as responders. Significant improvements were observed after risperidone treatment in the inattention, hyperactivity/impulsivity, ODD, and CD subscales of the Turgay DSM-IV-Based Child and Adolescent Behavior Disorders Screening and Rating Scale (parent and teacher forms). No severe adverse events were reported.Conclusions: The results of this study are consistent with previous findings and suggest that risperidone may be an effective and well-tolerated atypical antipsychotic drug for the treatment of children and adolescents with CD. However, further studies, particularly placebo-controlled and double-blinded, are needed to better define the clinical use of risperidone in children and adolescents with CD.